Successful Open Repair of a Thoracoabdominal Aortic Aneurysm After Multiple Failed Endovascular Treatments in a 22-Years-Old Individual With Loeys-Dietz Syndrome.

Annarita Santoro, Mohamed Rizk, Laura Inga Tavara, Moh'd Shafiq Ramadan, Germano Melissano
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Abstract

Loeys-Dietz syndrome is a rare genetically triggered disease characterized by aortic involvement, predisposing individuals to aneurysm and dissection at young age. Open repair is considered the treatment of choice despite the fact that it is associated with significant morbidity and mortality rates. On the other hand, endovascular treatment may be also considered an acceptable option in specific cases such as emergency or in patients unfit for open surgery or when landing zones are within surgical grafts. We report the case of a thoracoabdominal aortic aneurysm (TAAA) open surgical repair (OSR) in a 22-year-old male patient diagnosed with type 2 Loeys-Dietz syndrome, treated by means of a TAAA replacement with a 30-mm multi-branched "Coselli" aortic graft (Vascutek, Renfrewshire, Scotland, UK) after multiple previously interventions, including a thoracic endovascular aortic repair (TEVAR) and a custom made endograft for the visceral aorta.

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一名 22 岁的 Loeys-Dietz 综合征患者在多次血管内治疗失败后,成功开腹修补了胸腹主动脉瘤。
洛伊-迪茨综合征是一种罕见的由基因引发的疾病,其特点是主动脉受累,易在年轻时发生动脉瘤和夹层。尽管开胸修补术的发病率和死亡率较高,但仍被认为是首选治疗方法。另一方面,在特殊情况下,如急诊、不适合开放手术或着床区在手术移植物范围内的患者,血管内治疗也被认为是一种可接受的选择。我们报告了一例被诊断为 2 型 Loeys-Dietz 综合征的 22 岁男性胸腹主动脉瘤(TAAA)开放手术修复(OSR)病例,该病例采用 30 毫米多分支 "Coselli "主动脉移植物(Vascutek、英国苏格兰伦弗鲁郡)进行的 TAAA 置换治疗,此前曾进行过多次干预,包括胸腔内血管主动脉修复术(TEVAR)和定制的内脏主动脉内移植物。
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